Fig. 4.
Dose-dependent effect of vorinostat and panobinostat on multiple patient-derived NPC1 mutant cell lines. NPC1 mutant human fibroblasts were treated with vorinostat (A) or panobinostat (B) for 48 h, followed by fixation, staining with filipin, and imaging by using the ImageXpressMicro automatic fluorescence microscope. DMSO was used as a solvent control. Images were analyzed to obtain the LSO value as a measure of cholesterol accumulation. Data were normalized to the corresponding DMSO-treated cells. Data for each cell line are averages of three independent experiments totaling 60 images (5 wells × 4 sites × 3 experiments). Error bars show SEM.